Association between neutropenia and IgG antineutrophil antibodies in a case of CD40LG deficiency due to two novel mutations

Kristian Assing*, Kaspar René Nielsen, Helene Broch Tenstad, Marianne Antonius Jakobsen, Christian Nielsen, Dorthe Grosen, Ulla Birgitte Hartling

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Abstrakt

This case suggests a mechanistic rationale for the clinical efficacy of intravenous immunoglobulins (IVIG) in treating CD40 ligand (CD40L) deficiency associated neutropenia as it is the first reported instance of free and cell-bound antineutrophil antibodies in a case of CD40L deficiency, accompanied by a prolonged and clinically severe neutropenia.

OriginalsprogEngelsk
TidsskriftClinical Case Reports
Vol/bind8
Udgave nummer2
Sider (fra-til)313-316
ISSN2050-0904
DOI
StatusUdgivet - feb. 2020

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